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原发性系统性小血管炎的临床病理特点
引用本文:梁伟,熊祖应,廖瑾凤,陈丽,张帆,熊子波,罗琼.原发性系统性小血管炎的临床病理特点[J].罕少疾病杂志,2007,14(3):20-21.
作者姓名:梁伟  熊祖应  廖瑾凤  陈丽  张帆  熊子波  罗琼
作者单位:北京大学深圳医院肾内科,广东,深圳,518036
摘    要:目的 探讨原发性系统性小血管炎的临床病理特点和诊断,以提高对本病认识.方法 对2001年-2006年本院肾内科确诊的10例原发性系统性小血管炎临床资料进行分析,探讨原发性系统性小血管炎患者的临床病理特点.结果 10例患者男3例,女7例年龄24-77岁,平均55.4岁.3例ANCA阴性,7例IIF-ANCA阳性.10例均有肾脏损害,表现为血尿、蛋白尿、肾功能损害和病理改变.5例有肺受累,6例有听力损害.均有贫血,血沉增快和CRP明显升高.所有患者确诊时间均较长.结论 系统性小血管炎并不少见;临床表现呈多系统损害,肺、肾、血液系统及听力损害最常见;ANCA可作为诊断的特异性指标.

关 键 词:小血管炎  抗中性粒细胞胞浆抗体  临床病理  原发性系统性小血管炎  临床表现  病理特点  vasculitis  small  systemic  primary  特异性指标  血液系统  多系统损害  确诊时间  血沉增快  贫血  听力损害  受累  病理改变  肾功能损害  蛋白尿  血尿  肾脏损害
文章编号:1009-3257(2007)03-0020-02
修稿时间:2007-05-10

The clinicopathological characteristics of primary systemic small vasculitis
LIANG Wei. XIONG Zu-ying. LIAO Jin-lan,et al..The clinicopathological characteristics of primary systemic small vasculitis[J].Journal of Rare and Uncommon Diseases,2007,14(3):20-21.
Authors:LIANG Wei XIONG Zu-ying LIAO Jin-lan  
Institution:Department of Nephrology, Peking University Shenzhen Hospital, Guangdong, 518036
Abstract:Objective To investigate the clinicopathological characteristics and diagnosis of primary systemic small vasculitis. Methods The retrospective analysis of clinical materials was performed on 10 patients with primary systemic small vasculitis in our hospital from 2001 to 2007. Results Among the 10 patients, there were 3 male and 7 female.Their ages range from 24 to 77.The main clinicopathological characteristics included ANCA(antineutrophil cytoplasmic antibody) negative (3 cases),ANCA positive (7 cases),renal damage(10 cases) including haematuria,proteinuria,renal fuction damage and pathological damage,lung damage (5 cases), acouesthesia damage(6cases), anaemia(10 cases), rapid ESR and elevated CRP (10 cases), Conclusion Primary systemic small vasculitis is not rare in China. Its characteristics appears multi-system involvement,specially with lung, kidney,blood system and acouesthesia; ANCA can be used as a specific index for diagnosis.
Keywords:small vasculitis  antineutrophil cytoplasmic antibody  clinicopathology
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